Nine cases with arteriovenous malformations (AVM's) predominantly involving the lateral ventricle are presented. All the AVM's were small, but caused intraventricular hemorrhage in eight cases. Only two patients had an intracerebral hemorrhage large enough to warrant evacuation. Eight patients were under the age of 40 years at the onset of their disease. Computerized tomography demonstrated intraventricular hemorrhage in eight patients, and after intravenous administration of contrast medium a small area of enhancement with dilated subependymal draining veins was seen in seven. The lateral ventricles were of normal size in seven cases, and only two patients required a shunting procedure. Angiography demonstrated that the lesion was an AVM in eight patients, and did not visualize the lesion in the ninth. One patient suffered a recurrent intraventricular hemorrhage when the AVM was demonstrated, although repeated angiography had failed to disclose a vascular lesion at his first intraventricular hemorrhage 14 months before. All nine lesions were resected by microsurgical techniques, and the results were excellent in eight patients. Of four caudate lesions, three were resected through a frontal transcortical approach and the other was operated on through an anterior transcallosal approach; the results were excellent in three of these patients. Only one (Case 4) was left with neurological deficits; he had confusion and disorientation following a right frontal transcortical approach. Even in the dominant hemisphere, lesions in the head of the caudate nucleus could be safely resected by an anterior transcallosal approach. Two choroidal lesions located in the temporal horn and trigone on the dominant side were resected through a middle temporal gyrus approach, and three thalamic lesions through a posterior transcallosal approach, all with excellent results. In all cases the brain opening required was about the width of the retractor (maximum 2.0 cm, average 1.5 cm) 1).